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Self-Assessment Questions

Neurology

Familial Neurogenic Tumors: Review Questions

Bertrand C. Liang, MD

Dr.Liang is vice president of Oncology/Hematology, Biogen Idec Corporation, San Diego,  CA.



Choose the single best answer for each question.

1. Which of the following should be strongly considered as a possible etiology in this patient?
  1. Li-Fraumeni syndrome
  2. Neurofibromatosis type 1 (NF1)
  3. Neurofibromatosis type 2 (NF2)
  4. Tuberous sclerosis
  5. Turcot’s syndrome
Click here to compare your answer.


2. What other potential findings might be expected on physical examination of this patient given the possible etiology?

  1. Ashleaf spot
  2. Freckling in the axillary region
  3. Opacities in the posterior aspect of the lens
  4. Retinal angioma
  5. Skin pits on the palms and soles
Click here to compare your answer.


3. Which of the following familial tumor syndromes is associated with bilateral vestibular schwannomas?

  1. Li-Fraumeni syndrome
  2. NF1
  3. NF2
  4. Tuberous sclerosis
  5. Turcot’s syndrome
Click here to compare your answer.


  4. An infant is brought to the emergency department with a history of seizures manifest by flexor and extensor myoclonus. On physical examination, a hypomelanotic macule is noted on the infantıs back. Which of the following syndromes should be considered?
  1. Li-Fraumeni syndrome
  2. NF1
  3. NF2
  4. Tuberous sclerosis
  5. Turcot’s syndrome
Click here to compare your answer.


5. A 10-year-old boy enters the emergency department after having a tonic clonic seizure. His past medical history includes treatment for a rhabdomyosarcoma at age 6 years. A computed tomography scan of the brain shows an enhancing lesion with a necrotic center in the left frontoparietal lobe. Which of the following familial tumor syndromes should be considered?

  1. Cowden’s syndrome
  2. Gorlin’s syndrome
  3. Li-Fraumeni syndrome
  4. Tuberous sclerosis
  5. Turcot’s syndrome
Click here to compare your answer.


 

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